中线外周T细胞淋巴瘤19例临床分析

目的：分析探讨中线外周T细胞淋巴瘤（midline peripheral T-cell lymphoma,MPTL)的临床特点，诊断，分型，治疗方法的选择和预后。方法：经免疫组化证实的MPTL患者19例。病变累及一个解剖部位的局限型9例。其中8例单纯放射治疗，1例放疗加化疗的综合治疗；病变累及两个及两个以上解剖部位的广泛型10例，其中9例综合治疗，1例单纯化疗化疗方案为CCNU（环己亚硝脲）和CHOP（环磷酰胺。阿霉素，长春新碱，强的松）各5例。结果：19例MPTL患者3年生存率68.4%(13/19)。5年生存率55.6%(5/9)。其中局限型3年生存率88.9%(8/9)。广泛型3年生存率50.0%(5/10)。综合治疗CCNU方案3年生存2/5，CHOP方案3年生存4/5。结论：MPTL是以鼻面中线部位进行性破坏性病变为特征的恶性淋巴瘤。临床表现和病理形态呈现多样化，无特异性。免疫组化染色检查有助本病的诊断。依据病变侵犯的范围可将MPTL分为单一解剖部位受累的局限型和两个及两个以上解剖部位受累的广泛型，前者预后较好。可单纯放射治疗；后者采用放疗加化疗或化疗加放疗的综合治疗可提高远期生存率。辅助化疗以CCNU或CHOP方案为佳。

Clinical Analysis of 19 Cases of Midline Peripheral T-cell Lymphoma

Objective:To analyze the clinical characteristics of midline peripheral T-cell lymphoma (MPTL)and evaluate its diagnosis,classification,treatment and prognosis.Methods:19cases of MPTL confirmed by immunohistochemical stain were treated.9cases were local type involved in one anatomical site,8treated by radiotherapy only and1by combination of radio-therapy and chemotherapy.10cases were extensive type involved in two or more anatomical sites,9treated by combination therapy and1by chemotherapy only.As adjuvant chemotherapy,CCNU and CHOP(CTX,ADM,VCR,and PDN)regimens were given in5cases respectively.Results:The overall3-year and5-year survival rates were68.4%(13/19)and55.6%(5/9)respectively.The local and extensive type3-year survival rates were88.9%(8/9)and50%(5/10)respective-ly.The3-year survival rates of combination therapy with CCNU and CHOP regimens were2/5and4/5respectively.Conclusion:MPTL is a type of malignant lymphoma characterized by pro-gressive destructive lesions of nasal and facial midline structures.It has no specifications in the clinical manifestations and histopathologic features.Immunohistochemical stain is helpful to confirm the diagnosis of MPTL.MPTL can be divided into two types basing the extent of the lesion in-volvement,the local and extensive types.The local type is involved in one anatomical site and the extensive type is involved in two or more anatomical sites.The former can be treated by ra- diotherapy only with a better prognosis.The latter treated by radiotherapy+chemotherapy or chemotherapy+radiotherapy.The long-term survival rate of the extensive type can be enhanced with the combination of radiotherapy and chemotherapy,and a better therapeutic effect can be achieved treated by CCNU or CHOP regimen as the adjuvant chemotherapy.